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Disorders of adrenal gland

1. The document discusses disorders of the adrenal gland including anatomy, histology, functions, and various disorders affecting the adrenal cortex and medulla. 2. Key disorders covered include Cushing's syndrome, primary hyperaldosteronism, adrenocortical carcinoma, congenital adrenal hyperplasia, adrenal insufficiency, pheochromocytoma, neuroblastoma, and ganglioneuroma. 3. Diagnosis and treatment approaches are summarized for each disorder, including the role of surgery, particularly laparoscopic adrenalectomy.

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DISORDERS OF ADRENAL GLAND NUR AINA BINTI AB KADIR
OBJECTIVES  TO UNDERSTAND:  The anatomy and function of adrenal and other endocrine glands  The diagnosis and management  The role of surgery
ANATOMY  Weight = 4g  2 adrenal gland, right and left  2 component ; inner adrenal medulla and outer adrenal cortex  Situated near upper poles of kidneys in retroperitoneum, within Gerota’s capsule
ANATOMY  Right adrenal gland – between right liver lobe and diaphragm  Left adrenal gland – close to upper pole of left kidney and renal pedicle, covered by pancreatic tail and spleen
ANATOMY  Arterial blood supply –superior suprarenal artery (from inferior phrenic artery) -middle suprarenal artery (from abdominal aorta) - inferior suprarenal artery (from renal artery)
ANATOMY  Venous blood supply –Right suprarenal veins drain into inferior vena cava - Left suprarenal vein drain into left renal vein or left inferior phrenic vein
EMBRYOLOGY  Cortex and medulla arises from different blastomeric layers  Adrenal cortex arise from mesodermal cells  Adrenal medulla arise from neuroectodermal cells, which migrate to cortex from neural crest
HISTOLOGY  Adrenal cortex : Arranged in zonal configuration  Outer zona glomerulosa = small, compact cells  Central zona fasciculata = larger, lipid-rich cells arranged in radial columns  Inner zona reticularis = compact & pigmented cells  Adrenal medulla  Thin layers of large chromaffin cells
FUNCTION
FUNCTION
Incidentaloma Primary Hyperaldosteronism Cushing’s Syndrome Adrenocortical Carcinoma Congenital Adrenal Hyperplasia Adrenal Insufficiency DISORDERS OF ADRENAL CORTEX
INCIDENTALOMA  DEFINITION: a clinically unapparent mass detected incidentally by imaging studies conducted for other reasons.  INCIDENCE : 1.4%-8.7% , increase with age  DIAGNOSIS:  Hormone evaluation  Morning & midnight plasma cortisol measurements  1mg overnight dexamethasone suppresion test  24 h urinary cortisol excretion  12/24h urinary excretion of metanephrines / plasma-free metanephrines  Serum K+, plasma aldosterone & plasma renin activity  Serum DHEAS, testosterone/17-hyroxyestradiol  CT, MRI  Adrenal gland biopsy (to confirm metastasis)
TREATMENT  Non-functioning adrenal tumour > 4cm in diameter and smaller tumours that increase in size over time : surgical resection  Non-functioning tumour < 4 cm: followed-up after 6,12 and 24 months (imaging & hormonal evaluation)
PRIMARY HYPERALDOSTERONISM (PHA)  Hypertension , Hypokalemia, Hypersecretion of aldosterone  Hypertensive patient with hypokalaemic PHA~ 2%  Hypertensive patient have PHA with normal potassium : 12%
PRIMARY HYPERALDOSTERONISM (PHA)  Most : unilateral adrenocortical adenoma(Conn’s syndrome)  20-40%: bilateral micronodular hyperplasia  Rare : glucocorticoid- suppressible hyperaldosteronism/ adrenocortical carcinoma  Age : 30-50 years old with female predominance  Hypertension  Headache  Muscle weakness  Cramps  Intermittent paralysis  Polyuria  Polydypsia  Nocturia PATHOLOGY CLINICAL FEATURES
DIAGNOSIS  Biochemical test  Assess potassium and aldosterone level to plasma renin activity ratio  MRI / CT scan  To distinguish unilateral from bilateral disease  Conn’s adenomas usually measure 1-2cm  Selective adrenal vein catheterization Samples are obtained from vena cava and both veins Aldosterone to cortisol ratio (ACR) is determined in each sample
TREATMENT  1ST line therapy for PHA with bilateral hyperplasia: medical treatment- spironolactone  Most cases : supplemental antihypertensive medication  Unilateral laparoscopic adrenalectomy: clear evidence of unilateral/asymmetrical bilateral disease  Subtotal resection :typical Conn’s adenoma
CUSHING’S SYNDROME  Hypersecretion of cortisol caused by endogenous production/excessive use of corticosteroids
CLINICAL SYMPTOMS  Weight gain / central obesity  Diabetes  Hirsutism  Hypertension  Skin changes ( abd striae, facial plethora, ecchymosis, acne)  Muscle weakness  Depression / mania  Osteoporosis  Hypokalemia
DIAGNOSIS  Morning and midnight plasma cortisol levels are elevated  Dexamethasone fails to suppress 24-hour urinary cortisol excretion.  Serum ACTH level
Low ACTH level Detect pituitary tumor Detect ectopic cortisol producing tumor CT scan of chest and abdomen MRI positive Elevated ACTH level Performed MRI of pituitary gland MRI negative Assess adrenal gland Performed CT / MRI scan of adrenal gland
TREATMENT  Medical therapy with metyrapone or ketoconazole used in patients with severe hypercortisolism or if surgery is not possible.
MANAGEMENT PRE-OPERATIVE 1. Prophylactic anti- coagulation and the use of prophylactic antibiotics are essential 2. Cushing-associated diseases (diabetes, hypertension) must be controlled by medical theraphy POST-OPERATIVE 1. Supplemental cortisol should be given  15 mg h–1 is required parenterally for the first 12 hours followed by a daily dose of 100 mg for 3 days, which is gradually reduced thereafter.
ADRENOCORTICAL CARCINOMA  Rare malignancy with an incidence of 1-2 cases in 1mil population per year  Female predominance (1.5:1)  Age : 1st peak [ childhood], 2nd [ 4th & 5th decades]
ADRENOCORTICAL CARCINOMA  60% = cortisol excess (cushing’s syndrome)  Patient with non- functioning tumors  Abdominal discomfort  Back pain ( large tumors)  Measurement DHEAS, cortisol, catecholamines  Dexamethasone suppresion test  MRI/CT scan  MRI angiography (exclude tumor thrombus in VC)  CT scan of lung (distant metastases) CLINICAL PRESENTATION DIAGNOSIS
STAGING  McFarlane classification  Stage 1 : <5cm  Stage 2 : >5cm  Stage 3 : locally invasive tumour  Stage 4 : tumours with distant metastasis
TREATMENT  Complete tumor resection (R0)  En bloc resection with removal locally involved organ  Tumor debulking = control hormone excess  Tumor thrombus in vena cava = thrombectomy  Postoperatively :  Mitotane alone or in combination with etoposide, doxorubicin & cisplatin  Adjuvant radiotherapy = reduce local recurrence  Restaging for every 3 months = prevent relapse
CONGENITAL ADRENAL HYPERPLASIA (ADRENOGENITAL SYNDROME)  Virilisation and adrenal insufficiency in children  Autosomal recessive disorder caused by enzymatic defect in the synthetic pathway of cortisol & other steroid from cholesterol  95% : 21-hydroxylase deficiency  Common signs : hypertension & short stature  Treatment :  replacement cortisol + fludrocortisone  Large hypoplastic adrenals = remove (if symptomatic)
ADRENAL INSUFFICIENCY  DISEASES ASSOCIATED : polyglandular autoimmune syndroem, TB, after bilateral adrenalectomy, haemorrhage,metastases,etc
ADRENAL INSUFFICIENCY
TREATMENT  Immediate treatment must be started if patient displays features of adrenal insufficiency.  IV hydrocortisone 100mg every 6hrs, 3L of saline given in 6hrs under careful cardiac monitoring.  Chronic adrenal insufficiency = replacement therapy with daily oral hydrocortisone (10mg) and fludrocortisone (0.1mg)
PHAEOCHROMOCYTOMA(ADRENAL PARAGANGLIOMA) NEUROBLASTOMA GANGLIONEUROMA DISORDERS OF THE ADRENAL MEDULLA
PHAEOCHROMOCYTOMA  Tumour of the adrenal medulla, which is derived from chromaffin cells catecholamines  Sporadic occur after 4th decade, hereditary(earlier)  Known as 10% tumor  10% inherited  10% extra-adrenal  10% malignant  10% bilateral  10% children
AETIOLOGY  HEREDITARY PHAEOCHROMOCYTOMAS  Multiple endocrine neoplasia type 2(MEN 2)  Familial paraganglioma(PG) syndrome  Von Hippel-Lindau(VHL) syndrome  Neurofibromatosis (NF) type 1
PATHOLOGY  Greyish pink on the cut surface and usually highly vascularized  Area of haemorrhage & necrosis  M: polygonal but the configuration varies considerably
CLINICAL FEATURES
PHAEOCHROMOCYTOMA  DIAGNOSIS  Determination of adrenaline, noradrenaline, metanephrine, normetanephrine levels in a 24hour urine collection  Plasma-free metanephrine& normetanephrine  MRI- localisation/metastases  TREATMENT  Laparoscopic resection  >8-10cm/radiological signs of malignancy are detected open approach
PHAEOCHROMOCYTOMA MRI:’swiss cheese’ configuration 123I-MIBG (metaiodobenzyl - guanidine) single-photon emission computerised tomography (SPECT)
PHAEOCHROMOCYTOMA PREOPERATIVE  α- adrenoreceptor blocker (phenoxybenzamine)  Additional β- blockade PEROPERATIVE  Administration of pressor or vasodilator agents  Central venous catheter and invasive arterial monitoring POSTOPERATIVE  Observed patient 24 hours in ICU  Lifelong yearly biochemical tests should be performed
MALIGNANT PHAEOCHROMOCYTOMA  10% of phaeochromocytom a are malignant  Higher in paragangliomas  Metastatsis to lymph nodes, bone and liver  TREATMENT  Surgical excision  Tumor debulking  Symptomatic treatment by a- blockers  Mitotane(adjuvant& palliative treatment)  131I-MIBG/ combination chemotherapy
PHAEOCHROMOCYTOMA IN PREGNANCY  Amnion infection syndrome/pre eclampsia  1st and 2nd trimester : laparoscopic adrenalectomy after adequate a- blockade  Risk of miscarriage during surgery is high  3rd trimester: elective Caesarean with consecutive adrenalectomy
NEUROBLASTOMA  Malignant tumour that is derived from the sympathetic nervous system in the adrenal medulla (38%)/from any site along the paravertebral sites of abdomen(30%), chest(20%)  CLINICAL FEATURES  Newborn infants and young children < 5 years  Mass in abdomen, neck or chest  Proptosis/exophthalm us  Bone pain  Painless bluish skin metastases  Weakness or paralyses
PATHOLOGY  Pale and grey surface  Encapsulated  Typical area of calcification  With increased size of tumournecrosis and haemorrhage
NEUROBLASTOMA  DIAGNOSIS  Biochemical evaluation: urinary excretion(24hour urine) of VMA, HVA, dopamine, noradrenaline  CT/MRI of the chest& abdomen  Bone scan,BMA  MIBG scan  TREATMENT  Low-risk:Surgery (the addition of 6–12 w of chemotherapy-optional)  Intermediate- risk: surgery + multi-agent chemotherapy (carboplatin, cyclophosphamide, etoposide, doxorubicin)  High-risk patients: high- dose multi-agent chemotherapy followed by surgical resection in responding tumours and myeloablative stem cell rescue.
GANGLIONEUROMA  DEF: benign adrenal neoplasm that arises from NCT  Mature sympathetic ganglion cells & Schwann cells in fibrous stroma  CF: found in all age group(> before 60)  Occur anywhere along the paravertebral sympathetic plexus & in the adrenal medulla(30%)  Identified incidentally = CT/MRI for other indications  TREATMENT: surgical excision, laparoscopic
LAPAROSCOPIC ADRENALECTOMY: RIGHT ADRENALECTOMY LEFT ADRENALECTOMY RETROPERITONEOSCOPIC ADRENALECTOMY OPEN ADRENALECTOMY SURGERY OF THE ADRENAL GLANDS
INTRODUCTION  1990, laparoscopic/retroperitoneoscopic adrenalectomygold standard in the resection of tumor  Laparoscopic transperitoneal approach: better view of adrenal region  Small, bilateral tumours/in patients with hereditary tumour syndromes: subtotal resection  Open approach: radiological signs, dustant metastases, large tumors(>8-10cm)/distinct hormonal pattern suggest malignancy
RIGHT ADRENALECTOMY  Position: right side up,with table brake  Dissection start at the level of the periadrenal fat  Peritoneum is divided 2cm below the edge of liver from medial(IVC) to the lateral abdominal wall  Flap of peritoneum can be used to retract the liver up & off the adrenal  Identify the gland and mobilise gently, securing the vein with a clip/using one of the available energy device  Remove the gland in a plastic catch bag
LEFT ADRENALECTOMY  Position: right side  Mobilisation of the spleen will displace the pancreatic tail medially  Incison of Gerota’s fascia is followed by identification of the adrenal vein  The resection is completed by mobilising the adrenal gland at the level of the periadrenal fat  Remove the gland in a bag
RETROPERITONEOSCOPIC ADRENALECTOMY  1ST port: distal end of the 12th rib(prone position)  Digital dissection into the retroperitoneum, Gerota’s fascia is displaced ventrally  RAV is covered by the retrocaval posterior aspect of the adrenal gland  LAV is located at the medial inferior pole of the adrenal gland  High inflation pressures allow bloodless dissection effectively tamponading the veins
OPEN ADRENALECTOMY  Malignant adrenal tumour suspected  Rt side: hepatic flexure of the colon is mobilised & the rt liver lobe is cranially retracted to achieve an optimal exposure of the IVC & the adrenal gland  Lf side: AG can be exposed after mobilisation of the splenic flexure of the colon,through the transverse mesocolon/ the gastrocolic ligament  Resection of regional lymph node is recommended and should include resection of the tissue between the renal pedicle &
REFERENCES  Bailey & Love’s Short Practice of Surgery, 26th Edition,page778-787